Diagnosis/Classification Criteria for Behcet's Disease

Historical Background. The ISG criteria for Behcet's, created in 1990, have excellent specificity, but lack sensitivity. The International Criteria for Behcet's Disease (ICBD) was created in 2006, as replacement to ISG. The aim of this study was to compare their performance. ISG and ICBD Criteria. For ISG oral aphthosis is mandatory. The presence of any two of the following (genital aphthosis, skin lesions, eye lesions, and positive pathergy test) will diagnose/classify the patient as BD. For ICBD, vascular lesions were added, while oral aphthosis is no more mandatory. Getting 3 or more points diagnose/classify the patient as BD (genital aphthosis 2 points, eye lesions 2 points, and the remaining each one point). Performance and Comparison of ISG and ICBD. Their sensitivity, specificity, and accuracy (percent agreement), were tested in three independent cohort of patients from Far-East (China), Middle-East (Iran), and Europe (Germany). The sensitivity for ISG was respectively 65.4%, 78.1%, 83.7% and for ICBD 87%, 98.2%, and 96.5%. The specificity for ISG was 99.2%, 98.8%, 89.5% and for ICBD 94.1%, 95.6%, and 73.7%. The accuracy for ISG was 74.2%, 85.5%, 85.5% and for ICBD 88.9%, 97.3%, and 89.5%. Conclusion. ICBD has better sensitivity, and accuracy than ISG

Vitamin D deficiency in patients with Behcet’s disease

BACKGROUND: Behcet’s disease is an autoimmune, recurrent and multisystem disease. Vitamin D has immunomodulator role in immune system. So that vitamin D deficiency was reported in some autoimmune diseases. Behcet’s disease as a Silk Road disease is common in Iran. The aim of this study was to detect the serum level of 25(OH) vitamin D in Behcet’s patients and control group. METHODS: In this case–control study, 112 Behcet’s patients as cases group and 112 healthy individuals as controls group were enrolled. Any subject on vitamin D supplement, steroid, and immunosuppressors during the last 6 months were excluded. The serum level of 25(OH) vitamin D was measured in the two groups by ELISA method. The findings were compared via SPSS software. RESULTS: About 57% and 17% of Behcet’s patients had vitamin D deficiency and insufficiency respectively. Vitamin D deficiency was significantly more common in controls than cases group (P < 0.001). Vitamin D levels were significantly lower in controls (P < 0.001). Age and sex did not have any confounding effect on the results. There was no significant relationship between disease duration, disease activity, Pathergy test, HLA-B5, and HLA-B51 with vitamin D level in Behcet’s patients. CONCLUSIONS: Vitamin D deficiency is common among Behcet’s patients. However, our results revealed vitamin D deficiency was significantly more common in healthy controls in comparison with Behcet’s cases

Treatment of Behcet’s disease

Behcet’s disease is a systemic disease classified among vasculitides. Major manifestations are mucous membrane lesions (oral aphthosis and genital aphthosis), skin manifestations (pseudofolliculitis, erythema nodosum), ocular manifestations (uveitis, retinal vasculitis), joint manifestations, vascular lesions (small to large vessel thrombosis, aneurysm), gastrointestinal manifestations, orchiepididymitis, and some rare manifestations like cardiac, pulmonary, and renal impairment. Diagnosis is mainly clinical. The International Diagnosis Criteria for Behcet’s Disease may be of help. The gold standard of treatment for mucocutaneous lesions is colchicine. In refractory cases, levamisole, thalidomide, and dapsone may be of help. For major organ involvement like the eyes and the brain, immunosuppressive drugs and prednisolone are the gold standard. In refractory cases, biological agents are the last resort. For gastrointestinal manifestations, sulfasalazine and prednisolone are the first-line treatment. For vascular involvement, the first line treatment was anticoagulation, but recently it was shown that immunosuppressive drugs and prednisolone were confirmed to be the best. In all refractory cases and for all different organs, the last resort is biological agents

Fc-Gamma Receptor 3B Copy Number Variation Is Not a Risk Factor for Behçet's Disease

Behçet's disease (BD) is an immune-mediated systemic vasculitis associated with HLAB51. Other gene associations are likely and may provide further insight into the pathogenesis of this disease. Fc-gamma receptors play an important role in regulating immune function. Copy number variation (CNV) of the Fc-gamma receptor 3B (FCGR3B) gene is associated with other inflammatory conditions and may also play a role in BD. The aim of this study was to determine whether CNV of the FCGR3B gene is associated with BD or its clinical features. FCGR3B copy number was determined for 187 Iranian patients and 178 ethnicity-matched controls using quantitative real-time PCR. The genotype frequencies were comparable in both BD patients and controls. The odds ratio for low copy number (<2CN) was 0.6 (P = 0.16) and the odds ratio for high copy number (>2CN) was 0.75 (P = 0.50). There was no association found between high or low CN of the FCGR3B gene and BD or its clinical features in this Iranian population. We are the first to report this finding which, when looked at in the context of other genetic studies, gives us further insight into the complex pathogenesis of BD

Characterization of the major histocompatibility complex locus association with Behçet’s disease in Iran

© 2015 Xavier et al.; licensee BioMed Central. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.Introduction: The aim of this study was to characterize the association of human leukocyte antigen (HLA) B alleles and major histocompatibility complex (MHC) single nucleotide polymorphisms (SNPs) with Behçet's disease (BD) in an Iranian dataset. Methods: The association of three SNPs in the MHC region previously identified as the most associated in high-density genotyping studies was tested in a case-control study on 973 BD patients and 825 controls from Iran, and the association of HLA-B alleles was tested in a subset of 681 patients and 414 controls. Results: We found that HLA-B*51 (P = 4.11 × 10(-41), OR [95% CI] = 4.63[3.66-5.85]) and B*15 confer risk for BD (P = 2.83 × 10(-2), OR [95% CI] = 1.75[1.08-2.84]) in Iranian, and in B*51 negative individuals, only the B*15 allele is significantly associated with BD (P = 2.51 × 10(-3), OR [95% CI] = 2.40[1.37-4.20]). rs76546355, formerly known as rs116799036, located between HLA-B and MICA (MHC class I polypeptide-related sequence A), demonstrated the same level of association with BD as HLA-B*51 (P adj = 1.78 × 10(-46), OR [95% CI] = 5.46[4.21-7.09], and P adj = 8.34 × 10(-48), OR [95% CI] = 5.44[4.20-7.05], respectively) in the HLA-B allelotyped subset, while rs2848713 was less associated (P adj = 7.14 × 10(-35), OR [95% CI] = 3.73[2.97-4.69]) and rs9260997 was not associated (P adj = 1.00 × 10(-1)). Additionally, we found that B*51 genotype-phenotype correlations do not survive Bonferroni correction, while carriers of the rs76546355 risk allele predominate in BD cases with genital ulcers, positive pathergy test and positive BD family history (2.31 × 10(-4) ≤ P ≤ 1.59 × 10(-3)). Conclusions: We found that the HLA-B*51 allele and the rs76546355/rs116799036 MHC SNP are independent genetic risk factors for BD in Iranian, and that positivity for the rs76546355/rs116799036 risk allele, but not for B*51, does correlate with specific demographic characteristics or clinical manifestations in BD patients.This work was supported by the Portuguese Fundação para a Ciência e a Tecnologia (grants PTDC/SAU-GMG/098937/2008, PTDC/IIM-GES/5015/2012 and CMUP-ERI/TPE/0028/2013, fellowships SFRH/BD/43895/2008 to JMX, SFRH/BPD/35737/2007 to PA, SFRH/BPD/70008/2010 to IS, a Ciência and an Investigator-FCT contract to SAO), and the Research Committee of the Tehran University of Medical Sciences (grant 132/714).info:eu-repo/semantics/publishedVersio

Gluten sensitivity enteropathy in patients with recurrent aphthous stomatitis

<p>Abstract</p> <p>Background</p> <p>Gluten sensitive enteropathy (GSE) is an autoimmune enteropathy triggered by the ingestion of gluten-containing grains in susceptible individuals. Recurrent aphthous stomatitis (RAS) may be the sole manifestation of GSE. The aim of this study was to determine the prevalence of gluten sensitivity enteropathy (GSE) in a large group of patients with RAS and assess the efficacy of gluten free diet (GFD) on the improvement of aphthous lesions in those who were diagnosed with GSE.</p> <p>Methods</p> <p>Two hundred and forty seven patients with RAS were included. The patients had at least three aphthous attacks per year. Patients were screened by IgA anti-endomysial antibody (EMA), IgA anti tissue transglutaminase (TTG) and serum IgA level. Those with a positive serology underwent endoscopic biopsies of the duodenal mucosa and patients with negative serology were excluded. The diagnosis of GSE was based on a positive serological test and abnormal duodenal histology. For patients with GSE, gluten free diet was recommended.</p> <p>Results</p> <p>Six out of 247 RAS patients had positive TTG test alone, and one had positive EMA and TTG. All 7 patients with positive serologic tests underwent duodenal biopsies. Histological findings were compatible with GSE in all of them (Marsh I in four patients, Marsh II in two patients and Marsh IIIB in one another.). The mean age of GSE patients was 27.42 ± 10.56 (range, 13 to 40) years old. They were suffering from RAS for an average duration of 4.5 years. All of the 7 GSE patients had not responded to the routine anti-aphthae medications, including topical corticosteroids, tetracycline and colchicine. Four patients who adhered to a strict gluten-free diet showed noticeable improvement in their aphthous lesions over a period of 6 months.</p> <p>Conclusion</p> <p>A significant minority (e.g. 2.83%) of RAS patients have GSE. This could be compared with the 0.9% prevalence of GSE in the general population of Iran. This study suggests that evaluation for celiac disease is appropriate in patients with RAS. Additionally, the unresponsiveness to conventional anti-aphthae treatment could be an additional risk indicator.</p

Effect of early treatment in polymyositis and dermatomyositis

Background : Idiopathic inflammatory myopathies, dermatomyositis (DM) and polymyositis (PM) are rare but are potentially treatable. Aim : To compare the effect of early and late treatment in patients with PM and DM. Materials and Methods : The study included all the adult patients with definite diagnosis of PM or DM treated for at least 12 months. The patients were divided into two groups: Early Group - treatment within three months and Late Group - treatment after three months. The number of patients with positive therapeutic response, remission in less than one year and the mean time elapsed for reaching the remission were assessed and compared between the two groups. Chi-square test, Fisher′s exact test, t-test and Pearson correlation test were used for data analysis. Results : The analysis included 65 patients, 42 with DM and 23 with PM. Late Group included 24 patients (seven PM and 17 DM), while Early Group included 41 patients (16 PM and 25 DM). Positive therapeutic response, remission rate within one year was higher in Early Group (80% vs. 46%, P: 0.004). The mean time needed to achieve remission was much less with early treatment (5.5 vs. 11.9 months, P: 0.003). The relapse rate was also lower in Early Group (5% vs. 25%, P < 0.02). The comparison of treatment outcomes showed the same results in both PM and DM, but it was statistically significant in patients with DM. Conclusions : Early treatment in patients with PM and DM is associated with higher remission rates, shorter treatment period and low complication rates